Survival and outcome in 73 anti-Hu positive patients with paraneoplastic encephalomyelitis/sensory neuronopathy
| Autor: | Bertie de Leeuw, Charles J. Vecht, Wim van Putten, Herbert Hooijkaas, Joost Grefkens, Martin J. van den Bent, Peter A. E. Sillevis Smitt |
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| Přispěvatelé: | Neurology, Immunology |
| Rok vydání: | 2002 |
| Předmět: |
Male
medicine.medical_specialty Lung Neoplasms Nerve Tissue Proteins Neurological disorder Nervous System Gastroenterology Antibodies Central nervous system disease Predictive Value of Tests Internal medicine Antineoplastic Combined Chemotherapy Protocols medicine Humans Paraneoplastic Polyneuropathy Carcinoma Small Cell Survival rate Aged Autoantibodies Retrospective Studies Neuroradiology Neurologic Examination Neurons business.industry Hazard ratio Limbic encephalitis RNA-Binding Proteins Retrospective cohort study Middle Aged Prognosis medicine.disease Surgery Survival Rate Treatment Outcome ELAV Proteins Neurology Predictive value of tests Female Immunotherapy Neurology (clinical) business Paraneoplastic Syndromes Nervous System |
| Zdroj: | Journal of Neurology, 249, 745-753. D. Steinkopff-Verlag |
| ISSN: | 0340-5354 |
| Popis: | In a retrospective study, we determined clinical and serological findings, associated tumours, outcome and prognostic factors in 73 Hu-Ab positive patients detected in a Dutch reference laboratory. The most frequent signs and symptoms at presentation were sensory neuropathy (55 %), cerebellar degeneration (22 %), limbic encephalitis (15 %) and brainstem encephalitis (16 %). 23 % developed autonomic dysfunction including gastro-intestinal motility disorders in 14 %. In 85 % a tumour was detected, which was a lung tumour in 77 %. Signs, symptoms and associated tumours did not differ in six patients with additional neuronal antibodies (anti-amphiphysine, anti-CV2, anti-Ri). The overall 3 months, one-year and three-year survival rates from the time of diagnosis were 64 %, 40 % and 22 %. Rankin Scale Score (RS) at diagnosis and presence of tumour at the time of diagnosis predicted mortality with hazard ratios (95 % CI) of 2.6 (1.5-4.6) and 1.5 (1.1-2). The median delay between onset of symptoms and Hu-Ab diagnosis was 4 months. There was a negative association between delay RS at diagnosis (P=0.03). In a logistic regression analysis, only older age (OR=0.15; 0.02-0.63) and a higher RS at diagnosis (OR=0.29; 0.11-0.73) were associated with a lower probability of successful functional outcome. Adjusted for these factors, antitumour therapy showed a higher but statistically not significant probability of successful outcome (OR=3.5; 0.87-14.3). Our study underlines the importance of early diagnosis and start of antitumour treatment when the patient is still in a better functional state. The delay between onset of symptoms and diagnosis of PEM/SN suggests a window for improving outcome in these patients. |
| Databáze: | OpenAIRE |
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